International survey of T2* cardiovascular magnetic resonance in β-thalassemia major

John Paul Carpenter, Michael Roughton, Dudley J. Pennell, Taigang He, Paul Kirk, Lisa J. Anderson, V. John B. Porter, J. Malcolm Walker, Renzo Galanello, Fabrice Danjou, Gianluca Forni, Antonis Kattamis, Vassilis Ladis, Marouso Drossou, Demetra Vini, Andreas Michos, Vassilios Perifanis, Tuncay Hazirolan, Ana Almeida, Yesim AydinokSelen Bayraktaroglu, Mirella Rangelova, Denka Stoyanova, Valeria Kaleva, Georgi Tonev, Amal El-Beshlawy, Mohsen Elalfy, Ibrahim Al-Nasser, Wing Yan Au, Shau Yin Ha, Chi Kong Li, Winnie Chiu-Wing Chu, Lee Lee Chan, Meng Yao Lu, Lia Wahidiyat, Manuela Merelles-Pulcini, Juliano de Lara Fernandes, Fernando Kay, P. Joy Ho, Jane S. Hankins, Maria Cappellini, Shahina Daar, Jameela Sathar, Ali Taher, George Kontoghiorges, Khawla Belhoul, Alexis Thompson, Janet L. Kwiatkowski, Ru San Tan, Isabelle Thuret, Catherine Badens

Research output: Contribution to journalArticle

44 Scopus citations

Abstract

Accumulation of myocardial iron is the cause of heart failure and early death in most transfused thalassemia major patients. T2* cardiovascular magnetic resonance provides calibrated, reproducible measurements of myocardial iron. However, there are few data regarding myocardial iron loading and its relation to outcome across the world. A survey is reported of 3,095 patients in 27 worldwide centers using T2* cardiovascular magnetic resonance. Data on baseline T2* and numbers of patients with symptoms of heart failure at first scan (defined as symptoms and signs of heart failure with objective evidence of left ventricular dysfunction) were requested together with more detailed information about patients who subsequently developed heart failure or died. At first scan, 20.6% had severe myocardial iron (T2*≤10ms), 22.8% had moderate myocardial iron (T2* 10-20ms) and 56.6% of patients had no iron loading (T2*>20ms). There was significant geographical variation in myocardial iron loading (24.8-52.6%; P<0.001). At first scan, 85 (2.9%) of 2,915 patients were reported to have heart failure (81.2% had T2* <10ms; 98.8% had T2* <20ms). During follow up, 108 (3.8%) of 2,830 patients developed new heart failure. Of these, T2* at first scan had been less than 10ms in 96.3% and less than 20ms in 100%. There were 35 (1.1%) cardiac deaths. Of these patients, myocardial T2* at first scan had been less than 10ms in 85.7% and less than 20ms in 97.1%. Therefore, in this worldwide cohort of thalassemia major patients, over 43% had moderate/severe myocardial iron loading with significant geographical differences, and myocardial T2* values less than 10ms were strongly associated with heart failure and death.

Original languageEnglish (US)
Pages (from-to)1368-1374
Number of pages7
JournalHaematologica
Volume98
Issue number9
DOIs
StatePublished - Sep 1 2013

ASJC Scopus subject areas

  • Hematology

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    Carpenter, J. P., Roughton, M., Pennell, D. J., He, T., Kirk, P., Anderson, L. J., Porter, V. J. B., Malcolm Walker, J., Galanello, R., Danjou, F., Forni, G., Kattamis, A., Ladis, V., Drossou, M., Vini, D., Michos, A., Perifanis, V., Hazirolan, T., Almeida, A., ... Badens, C. (2013). International survey of T2* cardiovascular magnetic resonance in β-thalassemia major. Haematologica, 98(9), 1368-1374. https://doi.org/10.3324/haematol.2013.083634